sildenafil-citrate and Pleural-Effusion

The Fontan procedure is the final common pathway in the surgical palliation of many single ventricle heart defects. Mortality has improved but morbidity remains significant. Pleural effusions continue to present challenges after this operation and account for increased length of stay and increased time with draining tube thoracostomies. This review examines recent publications addressing this problem.. Four papers in 2008-2009 addressed effusions after the Fontan procedure. Off-pump Fontan procedures did not decrease time until chest tube removal. Pulmonary vascular compliance, derived from an electrical circuit model, predicted chest tube indwelling time. A retrospective study identified mean pulmonary artery pressure as a risk factor for effusions lasting more than 14 days after the Fontan procedure. One prospective, randomized trial evaluated the effects of lisinopril on pleural effusions after the Fontan procedure. There was no difference in the length of pleural drainage between the two groups.. Pleural effusions after the Fontan procedure continue to be a challenge. The cause is likely multifactorial and could explain why the literature has no clear message. One randomized, prospective trial suggests that fenestration reduces effusions. Many other reviews report no benefit to fenestration. Sildenafil after the Fontan procedure should be studied in a randomized, prospective fashion.

Topics: Antihypertensive Agents; Chest Tubes; Drainage; Fontan Procedure; Heart Defects, Congenital; Humans; Hypertension, Pulmonary; Lisinopril; Piperazines; Pleural Effusion; Postoperative Complications; Purines; Sildenafil Citrate; Sulfones; Vasodilator Agents

We evaluated whether the administration of sildenafil in children undergoing the TCPC operation shortened the interval from the operation to the removal of the pleural and peritoneal drains.. We retrospectively reviewed the data of 122 patients who had undergone the TCPC operation between 2004 and 2014. Patients were divided into two groups on the basis of their treatments. Sildenafil was orally administered pre-operatively in the morning of the procedure or within 24 hours after the TCPC operation to the sildenafil group (n = 48), which was compared to a control group (n = 60). Fourteen patients were excluded from the study.. The primary outcome measure was the time from the operation to the removal of the drains. The study groups had similar demographics. The median [interquartile range] time for the removal of drains (sildenafil group 11 [8-19] vs control group 11 [7-16] d, P = .532) was comparable between the groups. The median [interquartile range] fluid balance on the first post-operative day was significantly higher (P = .001) in the sildenafil group compared with controls (47 [12-103] vs 7 [-6-67] mL kg. Sildenafil administration, pre-operatively or within 24 hours after the TCPC operation, did not reduce the required time for pleural and peritoneal drains but was associated with a significantly higher positive fluid balance.

Topics: Ascitic Fluid; Child, Preschool; Female; Fontan Procedure; Heart Defects, Congenital; Humans; Male; Outcome Assessment, Health Care; Pleural Effusion; Retrospective Studies; Sildenafil Citrate; Time Factors; Water-Electrolyte Balance

We report a rare case of diffuse systemic sclerosis (SSc) evolving into diffuse SSc/systemic lupus erythematosus (SLE) overlap syndrome. A 15-year-old boy was diagnosed as diffuse SSc with initial presentations of Raynaud's phenomenon and skin tightening. He underwent Chinese herbal treatment and clinical symptoms deteriorated in the following 3 years. On admission to our ward, serositis with pleural effusion, severe pulmonary fibrosis with moderate pulmonary hypertension, swallowing difficulty, and polyarthritis were observed. Autoantibody profiles revealed concurrence of anti-double-stranded DNA, anti-Smith, anti-topoisomerase I, and anti-ribonucleoprotein antibodies. The patient fulfills the criteria for both diffuse SSc and SLE. After drainage for pleural effusion accompanied by oral prednisolone and sildenafil, there were improvement of respiratory distress, swallowing difficulty, and pulmonary hypertension. In conclusion, connective tissue diseases may overlap with each other during the disease course. Serial follow-up for clinical symptoms as well as serological changes is recommended.

Topics: Adolescent; Antihypertensive Agents; Arthritis; Autoantibodies; Biomarkers; Deglutition Disorders; Disease Progression; Drainage; Drugs, Chinese Herbal; Glucocorticoids; Humans; Hypertension, Pulmonary; Lupus Erythematosus, Systemic; Male; Piperazines; Pleural Effusion; Prednisolone; Pulmonary Fibrosis; Purines; Raynaud Disease; Scleroderma, Diffuse; Serositis; Sildenafil Citrate; Sulfones; Treatment Outcome